Chest infections/bronchitis/aspiration pneumonitis

Aetiology  

Multiple factors in adults with OA/TOF increase their prevalence of pulmonary morbidity. Sistonen et al. (2010) (14) found 56% of the adults aged between 22 and 56 had had pneumonia, 70% bronchitis and 52% reported recurrent respiratory infection. All below are strongly associated with recurrent pneumonia in repaired OA/TOF. (15)

• Oesophageal motility disorders/dysphagia
• Strictures
• GORD
• Anatomical anomalies (laryngeal cleft, vocal cord paralysis)

Symptoms

• Witnessed/known aspiration/choking event
• Fever
• Coughing
• Shortness of breath
• Raised heart and respiratory rate (16)

Diagnosis

This is easy when there is a witnessed episode of choking, but many result from ‘subclinical micro-aspiration and misidentification of chronic cough/wheeze and dyspnea’. (17) Diagnostic tools may include the following:

• Raised white cell count
• Chest X-ray – but changes aren’t necessarily visible
• Oesophageal manometry is useful to demonstrate the near universal disordered/absent peristalsis in those born with OA/TOF (18)
• CT, bronchoscopy and/or lung biopsy should be considered in patients with moderate-to-severe pulmonary morbidity due to airway reflux and aspiration episodes

Treatment

• Optimise management of airway reflux. (15)
• Antibiotics may not be needed if this is pneumonitis rather than aspiration pneumonia, but since one can’t differentiate clinically, empirical broad-spectrum antibiotics are usually advised. In addition, the difficulties clearing secretions from the airways can also allow bacterial infection to develop, so antibiotics are recommended in any episode lasting longer than two weeks. (6)
• Recurrent episodes or severe episodes of aspiration necessitate referral to a surgeon with knowledge of OA/TOF for consideration of fundoplication or tube feeding to protect the lungs.